Mortality in Adults With Sickle Cell Disease and Pulmonary Hypertension
Open accessAbstract
To the Editor: Dr Olshansky's research letter on the accelerated aging of US presidents concluded that presidents do not age faster than other men. 1 That may be true, but this study cannot demonstrate it due to a faulty comparison.Olshansky compared presidents' actual age at death (adjusted for aging by subtracting 2 days for every day in office) against average life expectancy for men matched to the date of inauguration.However, the correct comparison is with men who have the same profile of risk factors for premature death.For example, as Olshansky notes, education, wealth, and access to medical care strongly influence life expectancy, and these factors undoubtedly contributed to longer lives for many presidents.The real question is if, despite these advantages, presidents' lives were shortened.That requires comparison with other men of similar education, wealth, and access to medical care, and possibly with similar risk factors, such as smoking and alcohol consumption.Absent data permitting proper restriction, stratification, or control for confounding, the only conclusion that can be drawn is that presidents tend to be blessed with certain advantages that allow them to outlive men without those advantages.
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
Of these patients, 84 received right heart catheterizations due to suspected pulmonary hypertension, and 55 (10.4%) were diagnosed with pulmonary hypertension based on a mean pulmonary artery pressure >25 mm Hg.
“Of these patients, 84 received right heart catheterizations due to suspected pulmonary hypertension, and 55 (10.4%) were diagnosed with pulmonary hypertension based on a mean pulmonary artery pressure >25 mm Hg.”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [194]
The Results report that 84 of 531 patients underwent right heart catheterization and 55 had pulmonary hypertension, representing 10.4% of the total population. The Methods link catheterization to clinical suspicion but define pulmonary hypertension as mean pulmonary artery pressure of 25 mm Hg or greater, not greater than 25 mm Hg. (LLM, conf=0.90)
Experimental — accuracy not yet measuredThe NIH-PH cohort study screened 531 patients and followed them for a median of 4.4 years.
“The largest study with the longest follow-up is the NIH-PH cohort study, which screened 531 patients and followed them for a median of 4.4 years (66, 194).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [194]
Among the group with pulmonary hypertension, 56.4% had precapillary pulmonary arterial hypertension with a pulmonary artery occlusion pressure ≤15 mm Hg, and the remainder had pulmonary venous hypertension with elevated pulmonary artery occlusion pressures >15 mm Hg.
“Among the group with pulmonary hypertension, 56.4% had precapillary pulmonary arterial hypertension, with a pulmonary artery occlusion pressure ≤15 mm Hg, and the remainder had pulmonary venous hypertension, with elevated pulmonary artery occlusion pressures >15 mm Hg.”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [194]
Split into 2 atoms — 0 confirmed, 0 partially supported, 0 disputed, 0 misattributed, 2 unverifiable. Expand the claim to see per-atom verdicts.
Experimental — accuracy not yet measured
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Cites
Pulmonary Hypertension as a Risk Factor for Death in Patients with Sickle Cell Disease
Gladwin, M. T., Sachdev, V., Jison, M. L., Shizukuda, Y., Plehn, J. F., Minter, K., Brown, B., Coles, W. A., Nichols, J. S., Ernst, I., et al. (2004) · New England Journal of Medicine · doi:10.1056/nejmoa035477 · Cited by 1,325 · see citing works
Self-citationA Hemodynamic Study of Pulmonary Hypertension in Sickle Cell Disease
Parent, F., Bachir, D., Inamo, J., Lionnet, F., Driss, F., Loko, G., Habibi, A., Bennani, S. L., Savale, L., Adnot, S., et al. (2011) · New England Journal of Medicine · doi:10.1056/nejmoa1005565 · Cited by 511 · see citing works
Pulmonary hypertension in patients with sickle cell disease: a longitudinal study
Ataga, K. I., Moore, C. G., Jones, S., Olajide, O., Strayhorn, D., Hinderliter, A., & Orringer, E. P. (2006) · British Journal of Haematology · doi:10.1111/j.1365-2141.2006.06110.x · Cited by 298 · see citing works
Pulmonary hypertension diagnosed by right heart catheterisation in sickle cell disease
Fonsêca, G. H. H., Souza, R., Salemi, V. M. C., Jardim, C., & Gualandro, S. F. M. (2011) · European Respiratory Journal · doi:10.1183/09031936.00134410 · Cited by 273 · see citing works
Pulmonary hypertension and nitric oxide depletion in sickle cell disease
Bunn, H. F., Nathan, D. G., Dover, G. J., Hebbel, R. P., Platt, O. S., Rosse, W. F., & Ware, R. E. (2010) · Blood · doi:10.1182/blood-2010-02-268193 · Cited by 200 · see citing works
Flagged first, then by citations
Cited by
Sorted by citations
Updated clinical classification of pulmonary hypertension
Simonneau, G., Gatzoulis, M. A., Adatia, I., Celermajer, D., Denton, C., Ghofrani, A., Gomez Sanchez, M. A., Krishna Kumar, R., Landzberg, M., Machado, R. F., et al. (2013) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2013.10.029 · Cited by 4,191 · see citing works
Self-citation2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension
Humbert, M. J. C., Kovács, G., Hoeper, M. M., Badagliacca, R., Berger, R. M. F., Brida, M., Carlsen, J., Coats, A. J. S., Subías, P. E., Ferrari, P., et al. (2022) · European Heart Journal · doi:10.1093/eurheartj/ehac237 · Cited by 4,178 · see citing works
Haemodynamic definitions and updated clinical classification of pulmonary hypertension
Simonneau, G., Montani, D., Celermajer, D. S., Denton, C. P., Gatzoulis, M. A., Krowka, M., Williams, P. G., & Souza, R. (2019) · European Respiratory Journal · doi:10.1183/13993003.01913-2018 · Cited by 3,813 · see citing works
2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension
Humbert, M. J. C., Kovács, G., Hoeper, M. M., Badagliacca, R., Berger, R. M. F., Brida, M., Carlsen, J., Coats, A. J. S., Subías, P. E., Ferrari, P., et al. (2022) · European Respiratory Journal · doi:10.1183/13993003.00879-2022 · Cited by 2,335 · see citing works
Pediatric Pulmonary Hypertension
Abman, S. H., Hansmann, G., Archer, S. L., Ivy, D. D., Adatia, I., Chung, W. K., Hanna, B. D., Rosenzweig, E. B., Raj, J. U., Cornfield, D. N., et al. (2015) · Circulation · doi:10.1161/cir.0000000000000329 · Cited by 1,173 · see citing works
A global view of pulmonary hypertension
Hoeper, M. M., Humbert, M. J. C., Souza, R., Idrees, M. M., Kawut, S. M., Sliwa, K. S., Jing, Z., & Gibbs, J. S. R. (2016) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(15)00543-3 · Cited by 1,053 · see citing works
Pathophysiology of Sickle Cell Disease
Sundd, P., Gladwin, M. T., & Novelli, E. M. (2019) · Annual Review of Pathology Mechanisms of Disease · doi:10.1146/annurev-pathmechdis-012418-012838 · Cited by 813 · see citing works
Self-citationIntravascular hemolysis and the pathophysiology of sickle cell disease
Kato, G. J., Steinberg, M. H., & Gladwin, M. T. (2017) · Journal of Clinical Investigation · doi:10.1172/jci89741 · Cited by 748 · see citing works
Self-citationPediatric Pulmonary Hypertension
Ivy, D. D., Abman, S. H., Barst, R. J., Berger, R. M. F., Bonnet, D., Fleming, T. R., Haworth, S. G., Raj, J. U., Rosenzweig, E. B., Schulze‐Neick, I., et al. (2013) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2013.10.028 · Cited by 543 · see citing works
Kavanagh, P. L., Fasipe, T., & Wun, T. (2022) · JAMA · doi:10.1001/jama.2022.10233 · Cited by 507 · see citing works
Cardiovascular Abnormalities in Sickle Cell Disease
Gladwin, M. T., & Sachdev, V. (2012) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2011.10.900 · Cited by 275 · see citing works
Self-citationKlings, E. S., Machado, R. F., Barst, R. J., Morris, C. R., Mubarak, K. K., Gordeuk, V. R., Kato, G. J., Ataga, K. I., Gibbs, J. S. R., Castro, O. L., et al. (2014) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201401-0065st · Cited by 264 · see citing works
Self-citationPulmonary Complications of Sickle Cell Disease
Miller, A. C., & Gladwin, M. T. (2012) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201111-2082ci · Cited by 182 · see citing works
Self-citationPathophysiology and treatment of pulmonary hypertension in sickle cell disease
Gordeuk, V. R., Castro, O. L., & Machado, R. F. (2016) · Blood · doi:10.1182/blood-2015-08-618561 · Cited by 178 · see citing works
Self-citationCardiovascular complications and risk of death in sickle-cell disease
Gladwin, M. T. (2016) · The Lancet · doi:10.1016/s0140-6736(16)00647-4 · Cited by 164 · see citing works
Self-citationDifferences in the clinical and genotypic presentation of sickle cell disease around the world
Saraf, S. L., Molokie, R. E., Nouraie, M. S., Sable, C., Luchtman‐Jones, L., Ensing, G. J., Campbell, A., RANA, S. R., Niu, X. M., Machado, R. F., et al. (2013) · Paediatric Respiratory Reviews · doi:10.1016/j.prrv.2013.11.003 · Cited by 164 · see citing works
Self-citationDallas, M. H., Triplett, B. M., Shook, D. R., Hartford, C. M., Srinivasan, A., Laver, J. H., Ware, R. E., & Leung, W. H. (2013) · Transplantation and Cellular Therapy · doi:10.1016/j.bbmt.2013.02.010 · Cited by 154 · see citing works
Sickle Cell Anemia and Its Phenotypes
Williams, T. N., & Thein, S. L. (2018) · Annual Review of Genomics and Human Genetics · doi:10.1146/annurev-genom-083117-021320 · Cited by 151 · see citing works
Hemodynamic Predictors of Mortality in Adults with Sickle Cell Disease
Mehari, A., Alam, S., Tian, X., Cuttica, M. J., Barnett, C. F., Miles, G., Xu, D., Seamon, C., Adams-Graves, P., Castro, O. L., et al. (2013) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201207-1222oc · Cited by 135 · see citing works
Self-citationVasculopathy and pulmonary hypertension in sickle cell disease
Potoka, K. P., & Gladwin, M. T. (2014) · American Journal of Physiology-Lung Cellular and Molecular Physiology · doi:10.1152/ajplung.00252.2014 · Cited by 133 · see citing works
Self-citationGladwin, M. T., Barst, R. J., Gibbs, J. S. R., Hildesheim, M., Sachdev, V., Nouraie, M., Hassell, K. L., Little, J. A., Schraufnagel, D. E., Krishnamurti, L., et al. (2014) · PLoS ONE · doi:10.1371/journal.pone.0099489 · Cited by 132 · see citing works
Self-citationMinireview: Genetic basis of heterogeneity and severity in sickle cell disease
Habara, A. H., & Steinberg, M. H. (2016) · Experimental Biology and Medicine · doi:10.1177/1535370216636726 · Cited by 128 · see citing works
Sickle cell disease and venous thromboembolism: what the anticoagulation expert needs to know
Naik, R. P. P., Streiff, M. B., & Lanzkron, S. (2013) · Journal of Thrombosis and Thrombolysis · doi:10.1007/s11239-013-0895-y · Cited by 117 · see citing works
Sickle Cell Disease, Vasculopathy, and Therapeutics
Kassim, A. A., & DeBaun, M. R. (2012) · Annual Review of Medicine · doi:10.1146/annurev-med-120611-143127 · Cited by 113 · see citing works
Management of Patients with Sickle Cell Disease Using Transfusion Therapy
Chou, S. T., & Fasano, R. M. (2016) · Hematology/Oncology Clinics of North America · doi:10.1016/j.hoc.2016.01.011 · Cited by 112 · see citing works
Sparkenbaugh, E., Chantrathammachart, P., Mickelson, J., van Ryn, J., Hebbel, R. P., Monroe, D. M., Mackman, N., Key, N. S., & Pawliński, R. (2014) · Blood · doi:10.1182/blood-2013-08-523936 · Cited by 108 · see citing works
Sickle cell disease: Clinical presentation and management of a global health challenge
Houwing, M. E., de Pagter, P. J., van Beers, E. J., Biemond, B. J., Rettenbacher, E., Rijneveld, A. W., Schols, E. M., Philipsen, J. N. J., Tamminga, R. Y. J., van Draat, K. F., et al. (2019) · Blood Reviews · doi:10.1016/j.blre.2019.05.004 · Cited by 101 · see citing works
Cardiovascular complications of sickle cell disease
Sachdev, V., Rosing, D. R., & Thein, S. L. (2020) · Trends in Cardiovascular Medicine · doi:10.1016/j.tcm.2020.02.002 · Cited by 80 · see citing works
Sickle cell anemia mice develop a unique cardiomyopathy with restrictive physiology
Bakeer, N., James, J., Roy, S., Wansapura, J., Shanmukhappa, S. K., Lorenz, J. N., Osinska, H., Backer, K., Huby, A.-C., Shrestha, A., et al. (2016) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1600311113 · Cited by 79 · see citing works
The molecular basis for the prothrombotic state in sickle cell disease
Shet, A. S., Lizarralde-Iragorri, M. A., & Naik, R. P. P. (2020) · Haematologica · doi:10.3324/haematol.2019.239350 · Cited by 72 · see citing works
Orofacial Manifestations in Patients With Sickle Cell Disease
Javed, F., Correa, F. O., Almas, K., Nooh, N. S., Romanos, G. E., & Al‐Hezaimi, K. (2012) · The American Journal of the Medical Sciences · doi:10.1097/maj.0b013e318265b146 · Cited by 71 · see citing works
Sickle cell disease in the older adult
Thein, M. S., Igbineweka, N. E., & Thein, S. L. (2016) · Pathology · doi:10.1016/j.pathol.2016.10.002 · Cited by 66 · see citing works
Haematological determinants of cardiac involvement in adults with sickle cell disease
Damy, T., Bodez, D., Habibi, A., Guellich, A., Rappeneau, S., Inamo, J., Guendouz, S., Gellen-Dautremer, J., Pissard, S., Loric, S., et al. (2016) · European Heart Journal · doi:10.1093/eurheartj/ehv555 · Cited by 65 · see citing works
Mechanistic Insights and Characterization of Sickle Cell Disease–Associated Cardiomyopathy
Desai, A. A., Patel, A. R., Ahmad, H., Groth, J., Thiruvoipati, T., Turner, K. M., Yodwut, C., Czobor, P., Artz, N., Machado, R. F., et al. (2014) · Circulation Cardiovascular Imaging · doi:10.1161/circimaging.113.001420 · Cited by 65 · see citing works
Self-citationLiver injury is associated with mortality in sickle cell disease
Feld, J. J., Kato, G. J., Koh, C., Shields, T., Hildesheim, M. E., Kleiner, D. E., Taylor, J. G., Utay, N. S., Douek, D. C., Haynes–Williams, V., et al. (2015) · Alimentary Pharmacology & Therapeutics · doi:10.1111/apt.13347 · Cited by 64 · see citing works
Self-citationChronic Pulmonary Complications of Sickle Cell Disease
Mehari, A., & Klings, E. S. (2016) · CHEST Journal · doi:10.1016/j.chest.2015.11.016 · Cited by 62 · see citing works
Self-citationRuhl, A. P., Sadreameli, S. C., Allen, J. L., Bennett, D. P., Campbell, A., Coates, T. D., Diallo, D. A., Field, J. J., Fiorino, E. K., Gladwin, M. T., et al. (2019) · Annals of the American Thoracic Society · doi:10.1513/annalsats.201906-433st · Cited by 57 · see citing works
Self-citationSickle cell disease: time for a closer look at treatment options?
Sheth, S., Licursi, M., & Bhatia, M. (2013) · British Journal of Haematology · doi:10.1111/bjh.12413 · Cited by 57 · see citing works
Cardiovascular complications in patients with sickle cell disease
Gladwin, M. T. (2017) · Hematology · doi:10.1182/asheducation-2017.1.423 · Cited by 56 · see citing works
Self-citationAlterations of the Arginine Metabolome in Sickle Cell Disease
Morris, C. R. (2014) · Hematology/Oncology Clinics of North America · doi:10.1016/j.hoc.2013.11.008 · Cited by 54 · see citing works
Hematological disorders and pulmonary hypertension
Mathew, R., Huang, J. L., WU, J. M., Fallon, J. T., & Gewitz, M. H. (2016) · World Journal of Cardiology · doi:10.4330/wjc.v8.i12.703 · Cited by 52 · see citing works
Cardiovascular manifestations of sickle cell disease
Hammoudi, N., Lionnet, F., Redheuil, A. B., & Montalescot, G. (2019) · European Heart Journal · doi:10.1093/eurheartj/ehz217 · Cited by 52 · see citing works
How I treat the older adult with sickle cell disease
Thein, S. L., & Howard, J. (2018) · Blood · doi:10.1182/blood-2018-03-818161 · Cited by 51 · see citing works
Maron, B. A., & Abman, S. H. (2017) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201604-0882pp · Cited by 49 · see citing works
Hemoglobinopathies and sleep – The road less traveled
Gileles‐Hillel, A., Gozal, D., & Gozal, D. (2015) · Sleep Medicine Reviews · doi:10.1016/j.smrv.2015.01.002 · Cited by 46 · see citing works
Pulmonary Hypertension Associated with Chronic Hemolytic Anemia and Other Blood Disorders
Machado, R. F., & Farber, H. W. (2013) · Clinics in Chest Medicine · doi:10.1016/j.ccm.2013.08.006 · Cited by 45 · see citing works
Self-citationWang, X., Mendelsohn, L. G., Rogers, H. M., Leitman, S. F., Raghavachari, N., Yang, Y., Yau, Y. Y., Tallack, M. R., Perkins, A. C., Taylor, J. G., et al. (2014) · Blood · doi:10.1182/blood-2013-11-539718 · Cited by 45 · see citing works
Self-citationPulmonary Hypertension: NHLBI Workshop on the Primary Prevention of Chronic Lung Diseases
Austin, E. D., Kawut, S. M., Gladwin, M. T., & Abman, S. H. (2014) · Annals of the American Thoracic Society · doi:10.1513/annalsats.201312-443ld · Cited by 45 · see citing works
Self-citationSleep-disordered breathing and nocturnal hypoxemia in young adults with sickle cell disease
WHITESELL, P. L., Owoyemi, O., O’Neal, P., Nouraie, M. S., Klings, E. S., Rock, A., Mellman, T. A., Berihun, T., Lavela, J., Taylor, R. E., et al. (2016) · Sleep Medicine · doi:10.1016/j.sleep.2016.05.006 · Cited by 45 · see citing works
Sildenafil therapy in thalassemia patients with Doppler-defined risk of pulmonary hypertension
Morris, C. R., Kim, H.-Y., Wood, J. C., Porter, J. B., Klings, E. S., Trachtenberg, F. L., Sweeters, N., Olivieri, N. F., Kwiatkowski, J. L., Virzi, L., et al. (2013) · Haematologica · doi:10.3324/haematol.2012.082065 · Cited by 44 · see citing works
Measuring success: utility of biomarkers in sickle cell disease clinical trials and care
Kalpatthi, R. V., & Novelli, E. M. (2018) · Hematology · doi:10.1182/asheducation-2018.1.482 · Cited by 44 · see citing works
Olatunya, O. S., Lanaro, C., Longhini, A. L. F., Franco‐Penteado, C. F., Fertrin, K. Y., Adekile, A. D., Saad, S. T. O., & Costa, F. F. (2019) · Annals of Hematology · doi:10.1007/s00277-019-03792-x · Cited by 43 · see citing works
Pulmonary function, CT and echocardiographic abnormalities in sickle cell disease
Lunt, A. C., Desai, S. R., Wells, A. U., Hansell, D. M., Mushemi-Blake, S., Melikian, N., Shah, A. M., Thein, S. L., & Greenough, A. (2014) · Thorax · doi:10.1136/thoraxjnl-2013-204809 · Cited by 43 · see citing works
A Novel Molecular Signature for Elevated Tricuspid Regurgitation Velocity in Sickle Cell Disease
Desai, A. A., Zhou, T., Ahmad, H., Zhang, W., Mu, W., Trevino, S., Wade, M. S., Raghavachari, N., Kato, G. J., Peters-Lawrence, M. H., et al. (2012) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201201-0057oc · Cited by 41 · see citing works
Self-citationBallas, S. K. (2014) · European Journal Of Haematology · doi:10.1111/ejh.12460 · Cited by 39 · see citing works
Vascular complications of sickle cell disease
Usmani, A., & Machado, R. F. (2018) · Clinical Hemorheology and Microcirculation · doi:10.3233/ch-189008 · Cited by 38 · see citing works
Self-citationHoppe, C. C., & Neumayr, L. (2019) · Hematology/Oncology Clinics of North America · doi:10.1016/j.hoc.2019.01.014 · Cited by 38 · see citing works
Dense red blood cell and oxygen desaturation in sickle‐cell disease
Liberto, G. D., Kiger, L., Marden, M. C., Boyer, L., Canouï‐Poitrine, F., Conti, M., Rakotoson, M. G., Habibi, A., Khorgami, S., Vingert, B., et al. (2016) · American Journal of Hematology · doi:10.1002/ajh.24467 · Cited by 38 · see citing works
Sarode, R., Ballas, S. K., Garcia, A., Kim, H. C., King, K. E., Sachais, B. S., & Williams, L. A. (2016) · Journal of Clinical Apheresis · doi:10.1002/jca.21511 · Cited by 37 · see citing works
Pulmonary Hypertension in Sickle Cell Disease
Hayes, M. M., Vedamurthy, A., George, G., Dweik, R. A., Klings, E. S., Machado, R. F., Gladwin, M. T., Wilson, K. C., & Thomson, C. C. (2014) · Annals of the American Thoracic Society · doi:10.1513/annalsats.201408-405cme · Cited by 36 · see citing works
Self-citationPotoka, K. P., Wood, K. C., Baust, J., Bueno, M., Hahn, S. A., Vanderpool, R. R., Bachman, T. N., Mallampalli, G. M., Osei-Hwedieh, D. O., Schrott, V. M., et al. (2017) · American Journal of Respiratory Cell and Molecular Biology · doi:10.1165/rcmb.2017-0292oc · Cited by 35 · see citing works
Self-citationBarbosa, M. M., Vasconcelos, M. C. M., Ferrari, T. C. A., Fernandes, B. M., Passaglia, L. G., Silva, C. M., & Nunes, M. C. P. (2014) · Journal of the American Society of Echocardiography · doi:10.1016/j.echo.2014.07.014 · Cited by 33 · see citing works
Therapeutics Targeting of Dysregulated Redox Equilibrium and Endothelial Dysfunction
Risbano, M. G., & Gladwin, M. T. (2013) · Handbook of experimental pharmacology · doi:10.1007/978-3-642-38664-0_13 · Cited by 32 · see citing works
Self-citationProlongation of QT c intervals and risk of death among patients with sickle cell disease
Upadhya, B., Ntim, W. O., Stacey, R. B., Henderson, R., Leedy, D., O’Brien, F. X., & Knovich, M. A. (2013) · European Journal Of Haematology · doi:10.1111/ejh.12127 · Cited by 32 · see citing works
Dysregulated arginine metabolism and cardiopulmonary dysfunction in patients with thalassaemia
Morris, C. R., Kim, H., Klings, E. S., Wood, J. C., Porter, J. B., Trachtenberg, F. L., Sweeters, N., Olivieri, N. F., Kwiatkowski, J. L., Virzi, L., et al. (2015) · British Journal of Haematology · doi:10.1111/bjh.13452 · Cited by 32 · see citing works
Reduced sensitivity of the ferroportin Q248H mutant to physiological concentrations of hepcidin
Nekhai, S. A., Xu, M., Foster, A., Kasvosve, I., Diaz, S. F., Machado, R. F., Castro, O. L., Kato, G. J., Taylor, J. G., & Gordeuk, V. R. (2012) · Haematologica · doi:10.3324/haematol.2012.066530 · Cited by 31 · see citing works
Self-citationThe Burden of Pulmonary Hypertension in Resource-Limited Settings
Gidwani, S., & Nair, A. P. (2014) · Global Heart · doi:10.1016/j.gheart.2014.08.007 · Cited by 27 · see citing works
Paradoxical protection from atherosclerosis and thrombosis in a mouse model of sickle cell disease
Wang, H., Luo, W., Wang, J., Guo, C., Wolffe, S. L., Wang, J., Sun, E. B., Bradley, K., Campbell, A., & Eitzman, D. T. (2013) · British Journal of Haematology · doi:10.1111/bjh.12342 · Cited by 27 · see citing works
Riociguat use in sickle cell related chronic thromboembolic pulmonary hypertension: A case series
Weir, N. A., Conrey, A. K., Lewis, D., & Mehari, A. (2018) · Pulmonary Circulation · doi:10.1177/2045894018791802 · Cited by 27 · see citing works
Self-citationGroup 5 Pulmonary Hypertension
Kalantari, S., & Gomberg‐Maitland, M. (2016) · Cardiology Clinics · doi:10.1016/j.ccl.2016.04.004 · Cited by 26 · see citing works
Chaturvedi, S., Ghafuri, D. L., Kassim, A. A., Rodeghier, M., & DeBaun, M. R. (2017) · American Journal of Hematology · doi:10.1002/ajh.24598 · Cited by 25 · see citing works
Revisiting the hyperhemolysis paradigm
Gladwin, M. T. (2015) · Blood · doi:10.1182/blood-2015-06-649491 · Cited by 24 · see citing works
Self-citationRogers, N. M., Yao, M., Sembrat, J., George, M. P., Knupp, H. E., Ross, M., Sharifi‐Sanjani, M., Milošević, J., Croix, C. M. S., Rajkumar, R., et al. (2013) · Pulmonary Circulation · doi:10.1086/674754 · Cited by 24 · see citing works
Self-citationThe Cardiopulmonary Complications of Sickle Cell Disease
Desai, A. A., Machado, R. F., & Cohen, R. T. (2022) · Hematology/Oncology Clinics of North America · doi:10.1016/j.hoc.2022.07.014 · Cited by 24 · see citing works
Self-citationAllogeneic/Matched Related Transplantation for β-Thalassemia and Sickle Cell Anemia
Bernaudin, F., Pondarré, C., Galambrun, C., & Thuret, I. (2017) · Advances in experimental medicine and biology · doi:10.1007/978-1-4939-7299-9_4 · Cited by 24 · see citing works
Gonzales, J., Holbert, K., Czysz, K., George, J. T., Fernandes, C., & Fraidenburg, D. R. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23094763 · Cited by 23 · see citing works
How I treat hypoxia in adults with hemoglobinopathies and hemolytic disorders
Machogu, E. M., & Machado, R. F. (2018) · Blood · doi:10.1182/blood-2018-03-818195 · Cited by 23 · see citing works
Self-citationTherapeutics Targeting of Dysregulated Redox Equilibrium and Endothelial Dysfunction
Risbano, M. G., & Gladwin, M. T. (2013) · Handbook of Experimental Pharmacology · doi:10.1007/978-3-662-45805-1_13 · Cited by 32 · see citing works
Self-citationPittman, C., Hsieh, M. M., Coles, W. A., Tisdale, J. F., Weir, N. A., & Fitzhugh, C. D. (2017) · Bone Marrow Transplantation · doi:10.1038/bmt.2016.335 · Cited by 22 · see citing works
Allogeneic Hematopoietic Stem Cell Transplantation for Adults with Sickle Cell Disease
Saraf, S. L., & Rondelli, D. (2019) · Journal of Clinical Medicine · doi:10.3390/jcm8101565 · Cited by 22 · see citing works
Abnormal Ventilation–Perfusion Scan Is Associated with Pulmonary Hypertension in Sickle Cell Adults
Mehari, A., Igbineweka, N. E., Allen, D. T., Nichols, J., Thein, S. L., & Weir, N. A. (2018) · Journal of Nuclear Medicine · doi:10.2967/jnumed.118.211466 · Cited by 22 · see citing works
Self-citationEpidemiology, Pathogenesis, and Clinical Approach in Group 5 Pulmonary Hypertension
Al‐Qadi, M. O., LeVarge, B. L., & Ford, H. J. (2021) · Frontiers in Medicine · doi:10.3389/fmed.2020.616720 · Cited by 20 · see citing works
Dosunmu-Ogunbi, A. M., Yuan, S. S., Reynolds, M. J., Giordano, L., Sanker, S., Sullivan, M. L. G., Stolz, D. B., Kaufman, B. A., Wood, K. C., Zhang, Y., et al. (2021) · Blood · doi:10.1182/blood.2021013350 · Cited by 20 · see citing works
Machine‐learning algorithms for predicting hospital re‐admissions in sickle cell disease
Patel, A., Gan, K., Li, A. A., Weiss, J. C., Nouraie, M. S., TAYUR, S. R., & Novelli, E. M. (2020) · British Journal of Haematology · doi:10.1111/bjh.17107 · Cited by 19 · see citing works
High risk and low prevalence diseases: Acute chest syndrome in sickle cell disease
Koehl, J. L., Koyfman, A., Hayes, B. D., & Long, B. (2022) · The American Journal of Emergency Medicine · doi:10.1016/j.ajem.2022.06.018 · Cited by 19 · see citing works
Musa, B. M., Galadanci, N. A., Coker, M. O., Bussell, S. A., & Aliyu, M. H. (2016) · Annals of Hematology · doi:10.1007/s00277-016-2693-z · Cited by 19 · see citing works
Pulmonary Hypertension: Definition, Classification, and Diagnosis
Alves-Jr, J. L., Gavilanes, F., & Souza, R. (2017) · Seminars in Respiratory and Critical Care Medicine · doi:10.1055/s-0037-1606577 · Cited by 19 · see citing works
Ketamine for Sickle Cell Vaso-Occlusive Crises
Alshahrani, M. S. S., & Alghamdi, M. A. (2020) · Saudi Journal of Medicine and Medical Sciences · doi:10.4103/sjmms.sjmms_218_20 · Cited by 19 · see citing works
The role of phosphodiesterase inhibitors in the management of pulmonary vascular diseases
BUTROUS, G. S. (2014) · Global Cardiology Science and Practice · doi:10.5339/gcsp.2014.42 · Cited by 19 · see citing works
d’Humières, T., Savale, L., Inamo, J., Deux, J., Deswarte, S., Lionnet, F., Loko, G., Chantalat, C., Damy, T., Guillet, H., et al. (2021) · American Journal of Hematology · doi:10.1002/ajh.26271 · Cited by 18 · see citing works
Wood, K. C., Durgin, B. G., Schmidt, H. M., Hahn, S. A., Baust, J., Bachman, T. N., Vitturi, D. A., Ghosh, S., Ofori-Acquah, S. F., Mora, A. L., et al. (2019) · Blood Advances · doi:10.1182/bloodadvances.2019000621 · Cited by 18 · see citing works
Self-citationBilan, V. P., Schneider, F., Novelli, E. M., Kelley, E. E., Shiva, S., Gladwin, M. T., Jackson, E. K., & Tofovic, S. P. (2018) · Pulmonary Circulation · doi:10.1177/2045894018791557 · Cited by 17 · see citing works
Self-citationEtude de l’hémogramme dans la drépanocytose homozygote: à propos de 87 patients
Dahmanı, F., Benkirane, S., Kouzih, J., Woumki, A., Mamad, H., & Masrar, A. (2016) · Pan African Medical Journal · doi:10.11604/pamj.2016.25.240.11118 · Cited by 16 · see citing works
Shah, P., Suriany, S., Kato, R. M., Bush, A., Chalacheva, P., Veluswamy, S., Denton, C. C., Russell, K., Khaleel, M., Forman, H. J., et al. (2020) · American Journal of Hematology · doi:10.1002/ajh.26003 · Cited by 16 · see citing works
MicroRNA 648 Targets ET-1 mRNA and Is Cotranscriptionally Regulated withMICAL3by PAX5
Li, C., Gonsalves, C. S., Mpollo, M. E. M., Malik, P., Tahara, S. M., & Kalra, V. K. (2014) · Molecular and Cellular Biology · doi:10.1128/mcb.01199-14 · Cited by 16 · see citing works
Kang, B., Park, K., Kleinhenz, J., Murphy, T. C., Sutliff, R. L., Archer, D. R., & Hart, C. M. (2016) · American Journal of Respiratory Cell and Molecular Biology · doi:10.1165/rcmb.2016-0166oc · Cited by 16 · see citing works
Tavares, A. H. J., Benites, B. D., & Fertrin, K. Y. (2019) · Transfusion Medicine Reviews · doi:10.1016/j.tmrv.2019.04.001 · Cited by 16 · see citing works
Biological parameters predictive of percent dense red blood cell decrease under hydroxyurea
Rakotoson, M. G., Liberto, G. D., Audureau, É., Habibi, A., Fauroux, C., Khorgami, S., Lesne-Hulin, A., Loric, S., Pirenne, F., Galactéros, F., et al. (2015) · Orphanet Journal of Rare Diseases · doi:10.1186/s13023-015-0272-3 · Cited by 16 · see citing works
Pulmonary Hypertension in Sickle Cell Disease
Ristow, B., & Schiller, N. B. (2011) · New England Journal of Medicine · doi:10.1056/nejmc1109130 · Cited by 16 · see citing works
Sharma, S., Efird, J. T., Kadali, R. A. K., Mehra, S., Chohan, H., Daggubati, R. B., Liles, D., Gouge, C. A., Boettger, P., & Knupp, C. (2013) · Clinical Cardiology · doi:10.1002/clc.22153 · Cited by 15 · see citing works
- Authors
- Mehari, A., Gladwin, M. T., Tian, X., Machado, R. F., & Kato, G. J.
- Affiliations
- Howard University (US)
- Venue
- JAMA
- Publisher
- American Medical Association (AMA)
- Published
- 2012-03-28
- Volume
- 307
- Issue
- 12
- Pages
- 1254
- ISSN
- 0002-9955 (ISSN-L)
- 0098-7484
- PMC
- PMC3511048
- OpenAlex
- W1996335765
- Cited by
- 229
- References
- 5
- Citations by year
- 2026: 13 · 2025: 11 · 2024: 11 · 2023: 6 · 2022: 16 · 2021: 15 · 2020: 12 · 2019: 17 · 2018: 15 · 2017: 13 · 2016: 29 · 2015: 14 · 2014: 23 · 2013: 23 · 2012: 9
- FWCI
- 10.52
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- JAMA (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Funders
- Bayer Corporation
- Hemophilia Center of Western Pennsylvania
- National Institutes of Health (PO1-HL-103455)
- National Heart, Lung, and Blood Institute (R01HL096973, K23HL098454, ZIAHL006011, P01HL103455, ZIAHL006012, ZIAHL006015, R01HL098032)
- National Institute of Diabetes and Digestive and Kidney Diseases (RC1DK085852)
- NHLBI Division of Intramural Research
- Topics
- Health Sciences › Medicine › Genetics › Hemoglobinopathies and Related Disorders
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Keywords
- sickle cell disease, pulmonary hypertension, mortality, accelerated aging, American presidents, life expectancy, premature mortality
- MeSH terms
- Adult, Anemia, Sickle Cell, Echocardiography, Cardiac Catheterization, Humans, Hypertension, Pulmonary, Middle Aged, Retrospective Studies, Cohort Studies, Prevalence, Survival Analysis
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
